Neuroimaging Findings and Repeat Neuroimaging Value in Pediatric Chronic Ataxia

Background: Chronic ataxia, greater than two months in duration, is encountered relatively commonly in clinical pediatric neurology practise and presents with diagnostic challenges. It is caused by multiple and diverse disorders. Our aims were to describe the neuroimaging features and the value of repeat neuroimaging in pediatric chronic ataxia to ascertain their contribution to the diagnosis and management. Materials and Methods: A retrospective charts and neuroimaging reports review was undertaken in 177 children with chronic ataxia. Neuroimaging in 130 of 177 patients was also reviewed. Results: Nineteen patients had head computed tomography only, 103 brain magnetic resonance imaging only, and 55 had both. Abnormalities in the cerebellum or other brain regions were associated with ataxia. Neuroimaging was helpful in 73 patients with 30 disorders: It was diagnostic in 9 disorders, narrowed down the diagnostic possibilities in 14 disorders, and revealed important but non-diagnostic abnormalities, e.g. cerebellar atrophy in 7 disorders. Having a normal magnetic resonance imaging scan was mostly seen in genetic diseases or in the early course of ataxia telangiectasia. Repeat neuroimaging, performed in 108 patients, was generally helpful in monitoring disease evolution and in making a diagnosis. Neuroimaging was not directly helpful in 36 patients with 10 disorders or by definition the 55 patients with unknown disease etiology. Conclusions: Normal or abnormal neuroimaging findings and repeat neuroimaging are very valuable in the diagnosis and management of disorders associated with pediatric chronic ataxia. Résultats en neuro-imagerie et utilité d’examens répétés en neuro-imagerie dans les cas d’ataxie chronique chez l’enfant. Contexte: L’ataxie chronique, dont la durée excède deux mois, est un phénomène relativement fréquent dans la pratique clinique en neurologie pédiatrique et représente un défi en matière de diagnostic. Elle est causée par plusieurs différents types de désordres. Notre objectif est ici de décrire les divers aspects de la neuro-imagerie et l’utilité d’examens répétés en neuro-imagerie dans les cas d’ataxie chronique chez l’enfant, et ce, afin de déterminer leur apport en ce qui concerne le diagnostic et le suivi de cette pathologie. Matériel et méthodes: Nous avons passé en revue des récapitulatifs graphiques ainsi que des rapports en neuro-imagerie concernant 177 enfants atteints d’ataxie chronique. Les examens en neuro-imagerie de 130 p